Cardiac magnetic resonance imaging of congenital bicuspid aortic valves and associated aortic pathologies in adults

AIMS: Bicuspid aortic valve (BAV) represents the most frequent congenital cardiac abnormality resulting in premature valvular degeneration and aortic dilatation. In a large series of consecutive patients, we evaluated the distribution of BAV types and the associated valvular and aortic abnormalities. METHODS AND RESULTS: We investigated 266 patients (58 +/- 14 years) with BAV using a 1.5 T cardiac magnetic resonance (CMR) scanner. Valve morphology was described according to the Sievers classification. The aortic valve orifice area, aortic regurgitation (AR) fraction, and aortic dilation were quantified. Two hundred and forty-two data sets were available for analysis; 24% had BAV without a valvular lesion. The predominant valvular lesion was aortic stenosis (AS) with 51%. Lone AR was found in 17%. A combined lesion of AS and AR was found in 9%. Those with AS were older than the overall average (64 +/- 12 vs. 57 +/- 15 years, P < 0.001). The patients with AR and those without valvular abnormality were younger than average (49 +/- 13 and 50 +/- 12 years vs. 57 +/- 15 years, P < 0.01 respectively). Comparing two observers Kappa coefficient was 0.77 for differentiation of six valve morphologies and 0.80 for the differentiation of bicuspid and tricuspid valve. Aortic dilatation was found in 39% of cases with no discernible preference for any specific BAV-type and mainly affecting the ascending aorta. CONCLUSION: CMR can non-invasively differentiate various morphologies in BAV with low inter-observer variability. Valvular pathologies vary across age. Aortic dilatation is frequent in BAV independent from valvular morphology or lesion. In future CMR might help to guide management in patients with BAV.